Download Demyelinating Diseases

Extrapyramidal
diseases
Ⅰ.Formation of basal ganglia
*Corpus striatum:/caudate nucleus
\ neostriatum
\lentiform/putamen nucleus /
nucleus\globus pallidus- paleostriatum
*Red
nucleus
*Substantia
*Subthalamic
nigra
nucleus
Ⅱ Function
Regulation of voluntary motor
activity
Ⅲ.clinical features
1.Poverty of movement-hypertonia syndrome
position: substantia nigra ;globus pallidus
ex : Parkinson’ s disease
2.Increasing of movement-hypotonia syndrome
position: caudate nucleus ;putamen nucleus
ex : Chorea minor
Parkinson’s
Disease
Ⅰ.Definition
It is also called paralysis agitans. It
is a slowly progressive degeneration
disease of the extrapyramidal system. It
is characterized by tremor、rigidity、
bradykinesia 、abnormal gait and
attitude.
Ⅱ.Etiology and Pathogenesis
1.age:>40years old
\
2.Environmental factors :MPTP
3.Genetic factor
degeneration
of DA neurons
/
(>50%)
Ⅲ.Pathology and biochemical pathology
1.Pathology
(1)Macroscopic changes: no special changes
(2)Microscopic changes:widespread cellular
degeneration, in substantia nigra,
Lewy’s body
2.Biochemical pathology
HVA

tyrosine ↓
hydroxylase
dopadecar ↓ decomposition
- boxylase
↑MAO,COMT
Levo-tyrosine →→→→ Levodopa →→→→
dopamine ↓
↓↘
Caudate nucleus ←←←←←←←←←↓
↘
↙Substantia nigraAch ↑
putamen
↙ corpus striatum path
↓
nucleus
↓
clinical features
Ⅳ.Clinical Features
1.tremor:usually first occur(60-70%)
“pill-rolling”
static tremor : the tremor is present
when the patient is at rest. When the
patient is nervous ,the tremor
increase . When the patient move
voluntarily or sleep , the tremor is
suppressed or disappears entirely.
2.rigidity
Cog-wheel rigidity
lead-pipe rigidity
3.bradykinesia
(1).Voluntary movements are reduced
(2).Masked face
(3).micrographia
4.Abnormal gait and attitude
(1).generalized flexion
(2).festinating gait
5.Other symptoms
(1).autonomic disturbances :
hypersteatosis:oily face;
hyperhidrosis; constipation
sphincter disturbance is rare
(2).mental symptoms: dementia ;depression
(3).dysarthria
(4).salivation
Ⅴ.Diagnosis
1.It occurs in old people ,the onset is
insidious and progress gradually.
2.Clinical features: tremor 、rigidity 、
bradykinesia、abnormal attitude and gait .
Ⅵ.Differential Diagnosis
1.Secondary parkinson’s disease
(Parkinsonism)
(1).infection: encephalitis lethargica
(2).poisoning: CO,Mn
(3).drugs
(4).cerebral arteriosclerosis
(5).trauma
2.Others
(1).Depression
(2).Essential tremor
(3).multiple system atrophy: OPCA
(4).thyrotoxicosis,alcoholism.
Ⅶ.Treatment
1.Drug treatment
(1).anticholinergic drugs
Artane 2mg tid po;kemadrin 2.5mg tid po
Side-effects:
retention of urine;
enlarged pupil;
hypohidrosis; confusion
Contraindications:hypertrophy of prostrate
glaucoma
(2).amantadine: 50mg tid po
Side-effects :insomnia ; confusion;
hallucination
Contraindications: epileptic ;hepatic
and renal dysfunction
(3)Levodopa
L-dopa + dopadecarboxylase
inhibitor(DCI)
Ex : Madopar ; Sinemet
Side-effects
1.Peripheral side-effects:
nauea ;vomiting
Postural hypotension;
cardiac arrhythmia
2.Central side-effects
(1).motor fluctuation
a.End of dose deterioration
b.on-off phenomenon
(2).dyskinesia
a.peak-dose dyskinesia
b.Biphasic dyskinesia
c.Dystonia
(3).mental symptoms:confusion;hallucination
(4)Dopamine receptor stimulant drug:
bromocriptine
(5)monoamine oxidase B inhibitor
(6)COMT inhibitor
2.Surgical treatment
3.Cell transplantation and gene therapy
4.Rehabilitation treatment
Chorea
minor
Ⅰ.Definition
It is also called Sydenham chorea. It is
a common nervous system manifestation of
rheumatic fever, it often occurs in
children . It is characterized by
involuntarily choretic movements、
hypotonia、weakness、 mental symptoms.
Ⅱ Etiology
Infection of A hemolytic
streptococus
Ⅲ.Clinical features
1.onset:5-15 years old ; F>M; subacute
or insidious
2.Choretic movements
3.Muscular tension and power are reduced
4.Mental symptoms
5.Symptoms of rheumatic fever: cardiac
disease; fever; WBC↑;rheumatic
arthritis; subcutaneous nodule ;blood
sedimentation↑;ASO↑
Ⅳ.Diagnosis
1.The age of onset
2.Clinical features
3.Symptoms of rheumatic fever
Ⅴ.Treatment
1.Etiological treatment
Penicillin 10-14 days
2.Symptomatic treatment
Valii; luminal; chlorpromazine
Hepatolenticular Degeneration
(HLD)
Ⅰ.Definition
It is also called Wilson’s disease.It
is an autosomal recessive inheritant
disease caused by copper metabolic
disorder. The affected areas mainly
are liver and basal ganglia. It is
characterized by
progressive
extrapyramidal symptoms、cirrhosis of
the liver 、mental symptoms and K-F
ring.
Ⅱ.Etiology and Pathogenesis

Copper  + 2 globulins  ceruloplasmin enzyme

 bile,urine,sweet


 liver cirrhosis of the liver
abnormal  basal ganglia  extrapyramidal
deposition
symptoms
 kidney  renal dysfunction
 cornea  K-F ring
Ⅲ.Pathology
Degeneration: liver
basal ganglia
kidney
cornea
Ⅳ.Clinical Features
1.nervous symptoms
(1)extrapyramidal symptoms: choretic
action;tremor;rigidity;bradykinesia
(2)mental symptoms:intelligent deficiency
2.symptoms of cirrhosis of the liver
3.K-F ring: 95%
4.others: renal dysfunction
Ⅴ.Investigation
1.ceruloplasmin of serum:↓0.26-0.36g/L
2.copper oxidase activity:↓
Ⅵ.Diagnosis
1.the symptoms of liver and
extrapyramidal system.
2.the levels of CP and copper
oxidase activity are reduced.
3.K-F ring of cornea
4.positive family history
Ⅶ.Treatment
1.low-copper diet
2.drug treatment
first -choice: D-penicillamine
3.symptomatic therapy
4.surgical treatment